1/134. Fine needle aspiration diagnosis of extraadrenal myelolipoma presenting as a pleural mass. A case report.BACKGROUND: myelolipoma is a benign tumor composed of mature adipose tissue and hematopoietic cells. Although they are commonly found in adrenal glands, extraadrenal myelolipomas (EMLs) are rare but well documented. They have been found in various sites, including mediastinum, liver, stomach, lungs, pelvis, spleen, retroperitoneum, presacral region and mesentery. EMLs must be distinguished from extramedullary hematopoieses, which are also composed of hematopoietic elements but may lack adipose tissue and are associated with anemia and marked bone marrow hyperplasia. CASE: We describe a case of a pleura-based, extraadrenal myelolipoma in a 53-year-old female with unremarkable bone marrow findings that were initially encountered on fluoroscopy-guided fine needle aspiration (FNA). One year later the mass was removed via open thoracotomy. It showed typical EML features histologically. CONCLUSION: EML manifests on aspiration cytology as a cellular specimen with numerous trilineage hematopoietic cells and a variable proportion of mature adipose cells. To our knowledge, FNA cytology of EML has not been found in this location before. Aspiration biopsy offers a simple and reliable method for the diagnosis of EML in the presence of appropriate clinical settings.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
2/134. Posttraumatic hypertension secondary to adrenal hemorrhage mimicking pheochromocytoma: case report.We report the case of a 68-year-old man who presented with a mass 3 x 4 cm in size located in the right adrenal gland together with extreme hypertension, tripled urine levels for normetanephrine, and normal plasmatic levels of metanephrines. The patient had suffered a fall from a height of 2.5 meters before hospitalization. [123I]MIBG-scan was repeatedly positive in the area of the right adrenal gland. At laparotomy under alpha-adrenergic blocking agents, the suspected pheochromocytoma was histologically confirmed as hematoma. After resection of the adrenal gland, blood pressure returned to normal without drug therapy as did metanephrine levels in urine. Although adrenal insufficiency after distension of the gland caused by hemorrhage has been reported, there are no data available regarding the mimicking of a hormonally active pheochromocytoma. We conclude that intra-adrenal pressure rise caused by hematoma may cause partial ischemic necrosis to the gland but may also induce reactive hyperplasia with periodic excessive secretion of catecholamines. This interpretation is consistent with the finding that plasma levels of catecholamines were normal in contrast to the urinary normetanephrines in the presented case. It might be worthwhile to investigate patients with intra-adrenal hemorrhage immediately after sustaining multiple injuries and in the posttraumatic course of several months up to 1 or more years together with verification of abnormal urinary excretion of metanephrines as a sign of impaired adrenal function.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
3/134. A case of aldosterone-producing adenoma with severe postoperative hyperkalemia.It is known that some patients with primary aldosteronism show postoperative hyperkalemia, which is due to inability of the adrenal gland to secrete sufficient amounts of aldosterone. However, hyperkalemia is generally neither severe nor prolonged, in which replacement therapy with mineralocorticoid is seldom necessary. We report a case of a 46-year-old woman with an aldosterone-producing adenoma associated with severe postoperative hyperkalemia. After unilateral adrenalectomy, the patient showed episodes of severe hyperkalemia for four months, which required not only cation-exchange resin, but also mineralocorticoid replacement. plasma aldosterone concentration (PAC) was low, although PAC was increased after rapid ACTH test. Histological examination indicated the presence of adrenocortical tumor and paradoxical hyperplasia of zona glomerulosa in the adjacent adrenal. immunohistochemistry demonstrated that the enzymes involved in aldosterone synthesis, such as cholesterol side chain cleavage (P-450scc), 3beta-hydroxysteroid dehydrogenase (3beta-HSD), and 21-hydroxylase (P-450c21), or the enzyme involved in glucocorticoid synthesis, 11beta-hydroxylase (P-450c11beta), were expressed in the tumor, but they were completely absent in zona glomerulosa of the adjacent adrenal. These findings were consistent with the patterns of primary aldosteronism. serum potassium level was gradually decreased with concomitant increase in PAC. These results suggest that severe postoperative hyperkalemia of the present case was attributable to severe suppression of aldosterone synthesis in the adjacent and contralateral adrenal, which resulted in slow recovery of aldosterone secretion. It is plausible that aldosterone synthesis of adjacent and contralateral adrenal glands is severely impaired in some cases with primary aldosteronism, as glucocorticoid synthesis in cushing syndrome.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
4/134. hypertension and virilization caused by a unique desoxycorticosterone- and androgen-secreting adrenal adenoma.We describe a rare androgen and desoxycorticosterone (DOC)-secreting adrenal tumor in a non-Cushingoid 14 year-old Haitian girl with secondary amenorrhea, hypertension and virilization. Her steroid pattern simulated an 11 beta-hydroxylation defect with notable elevation of adrenal androgens, 11-desoxycortisol (S), DOC, 17 alpha-hydroxyprogesterone and pregnenelone. Exogenous ACTH stimulated steroidogenesis. A CAT scan unfortunately failed to delineate an adrenal mass. dexamethasone (DEX) was administered, therefore, which partially suppressed androgen levels, reduced DOC and S by 80% and 82% respectively, and normalized blood pressure. Nevertheless, the response to glucocorticoid was incomplete and an MRI was obtained, which revealed a right adrenal tumor. Post surgery, the patient promptly resumed menses and became normotensive. This case illustrates that ACTH and DEX cannot reliably differentiate tumor from hyperplasia, whereas the simultaneous increase of delta 4 and delta 5 steroids, present here, may favor a tumor. This case also allows speculation that the hypersecretion of DOC may result from inhibition of 11 beta-hydroxylase activity by excess androgens. The importance of appropriate imaging for diagnosis is underscored.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
5/134. pheochromocytoma and sub-clinical Cushing's syndrome during pregnancy: diagnosis, medical pre-treatment and cure by laparoscopic unilateral adrenalectomy.The coexistence of pheochromocytoma and primary adrenal Cushing's syndrome of the same adrenal gland has rarely been reported. We describe here the case of a female patient presenting with mild Cushing's stigmata, hypertension and diabetes mellitus in whom we diagnosed a pheochromocytoma of the left adrenal gland with coexisting non-ACTH-dependent cortisol hypersecretion. While hormonal work-up was still in progress, the patient became pregnant and wanted to carry her pregnancy to full-term. A laparoscopic adrenalectomy in the 17th week of gestation was decided upon and the patient accordingly prepared for surgery by pre-treatment with phenoxybenzamine. Successful surgery--the first ever reported laparoscopic resection of a pheochromocytoma in pregnancy--without perioperative complications was performed under general anesthesia, with the patient receiving peri- and post-operative hydrocortisone substitution. Pathohistological examination revealed a pheochromocytoma with positive immunostaining for interleukin-6 (IL-6) and negative immunostaining for ACTH, vasoactive intestinal polypeptide (VIP) and cytochrome P450, and with no signs of malignancy. A paracrine stimulation of the ipsilateral adrenal cortex by IL-6 produced by the pheochromocytoma, leading to cortical hyperplasia and subclinical Cushing's syndrome, is suggested by the positive immunostaining for IL-6 and the MRI findings. Post-operatively, secondary adrenal insufficiency ensued, necessitating continuing hydrocortisone replacement over 12 months. hypertension resolved after surgery, and diabetes after the uncomplicated vaginal delivery at term.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
6/134. Fine-needle aspiration cytology of primary granulosa cell tumor of the adrenal gland: a case report.Extraovarian granulosa cell tumors are extremely rare. We report on a primary granulosa cell tumor of the adrenal gland. A 69-yr-old African-American female presented with a 1-yr history of irregular uterine bleeding and a palpable right abdominal mass. CT scan showed a 9.0-cm suprarenal mass as well as an enlarged uterus. CT-guided fine-needle aspiration (FNA) cytology of the adrenal mass was interpreted as a malignant neoplasm. She underwent exploratory laparotomy, right nephrectomy, and hysterectomy with bilateral salpingo-oophorectomy. The gross, histologic, and immunohistochemical findings of the adrenal mass were characteristic of a granulosa cell tumor. The uterus contained multiple leiomyomas. The endometrium showed simple hyperplasia. Both fallopian tubes and ovaries showed no pathologic abnormality. There was no evidence of tumor elsewhere. Although rare, extraovarian granulosa cell tumor should be considered in the differential diagnosis of adrenal tumors in women showing the FNA features described herein, especially when there is evidence of excessive estrogen production. Diagn. Cytopathol. 2000;22:107-109.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
7/134. multiple endocrine neoplasia type 2a. Study of a family.INTRODUCTION: Pheochromocytomas (Pheo) can occur sporadically, isolated or in association with other neuroendocrine lesions. In multiple endocrine neoplasia type 2a (MEN-2A), Pheo is associated to medullary thyroid carcinoma (MTC) or its precursor, C-cell hyperplasia (CCH) and parathyroid hyperplasia. Genetic screening provides early diagnosis and preventive treatment. In order to validate dna analysis as a reliable method of early identification of gene carriers, we compared the results of genetic screening with clinical, biochemical, imaging and pathological findings in the members of an affected family. population AND methods: The diagnosis of a bilateral necrotic Pheo in a female patient led to the study of a family with four generations, aged 3 to 78 years (mean = 30.3 yrs). The study included a clinical examination; basal and pentagastrin stimulated calcitonin values; urinary catecholamines and their metabolites; serum calcium and a genetic study (direct sequence of PCR products from genomic dna isolated from leucocytes using specific primers in exon 11 of the RET protooncogene of chromosome 10). The radiologic study, gammagraphic study (131I-MIBG) and magnetic resonance study were performed in members with clinical suspicion of Pheo. RESULTS: Seven out of nine patients had a mutation on codon 634 of exon 11 of RET (TGC-CGC), leading to cysteine arginine substitution in the codified protein; all gene carriers had biochemical markers of MTC/CCH and four of Pheo. The Pheo patients underwent adrenalectomy (bilateral in three) and all the gene carriers underwent prophylactic thyroidectomy. The pathologic findings were: MTC in four (metastasized in one); CCH in three and parathyroid hyperplasia in one. CONCLUSIONS: Phenotypic penetration of RET mutation was 100% for MTC/CCH, but only 57% of the gene carriers had Pheo. Genetic screening allowed early prophylactic treatment in four out of seven patients; pathologic findings revealed several evolutionary stages of the disease. patients not yet showing Pheo are under close clinical and laboratory surveillance.- - - - - - - - - - ranking = 3keywords = hyperplasia (Clic here for more details about this article) |
8/134. Subclinical cushing syndrome due to adrenocorticotropic hormone-independent macronodular adrenocortical hyperplasia: changes in plasma cortisol levels during long-term follow-up.A 63-year-old man with bilateral adrenal tumors was treated for hypertension and diabetes mellitus. Endocrinologic examination during follow-up revealed the production of cortisol to be independent of the regulation of the hypothalamo-pituitary-adrenal axis. Therefore, he was diagnosed as having subclinical cushing syndrome, and these tumors were removed. Adrenocorticotropic hormone-independent macronodular adrenal hyperplasia (AIMAH) was endocrinologically and histopathologically diagnosed. This is the first report demonstrating that endocrinologic changes of AIMAH can be observed during long-term follow-up.- - - - - - - - - - ranking = 5keywords = hyperplasia (Clic here for more details about this article) |
9/134. Large bilateral adrenal incidentalomas complicating untreated 11B hydroxylase deficiency in the third decade of life. A case report.The occurrence of bilateral giant adrenal incidentalomas is reported in a 22-year-old man who was evaluated for urinary tract infection. The right gland measured 16 cm, the left one 6 cm at computed tomography. Height was 145 cm, blood pressure 190/100 mm Hg. Testes were not palpable. Laboratory investigations revealed elevated levels of 17 hydroxyprogesterone:>50 ng/ml (n<1,1); 11 desoxycortisol: 919 nmol/l (n<30); testosterone: 19 ng/ml (n<0.7) and ACTH: 1 402 ng/l (n<48). karyotype was 46 XX. The patient was a female pseudohermaphrodite with congenital adrenal 11 B hydroxylase deficiency. Adrenal masses responded to glucocorticoid therapy with marked reduction of their size after six months. We confirm previous recommendations that patients with adrenal incidentaloma should be checked for congenital adrenal hyperplasia.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
10/134. Molecular characterization of a pediatric pheochromocytoma with suspected bilateral disease.An 11-year-old boy with hypertension was suspected of having bilateral adrenal pheochromocytomas and hyperplasia. Molecular analysis of specific tumor suppressor genes and oncogenes excluded the familial syndromes, von Hippel-Lindau (VHL) disease and multiple endocrine neoplasia (MEN) type 2A. Further evaluation identified a unilateral adrenal pheochromocytoma with a VHL heterozygous somatic mutation (G695A) and loss of the maternal allele at 11p15.5-11p14 exclusively in the tumor tissue. Both reverse-transcriptase polymerase chain reaction and immunohistochemistry confirmed increased expression of IGF2 within the tumoral tissue, relative to a normal control adrenal gland. These results ruled out familial syndromes and suggested that the VHL mutation and the loss of maternal allele on chromosome 11 could have contributed to tumor development.- - - - - - - - - - ranking = 1keywords = hyperplasia (Clic here for more details about this article) |
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